gms | German Medical Science

102. Jahrestagung der DOG

Deutsche Ophthalmologische Gesellschaft e. V.

23. bis 26.09.2004, Berlin

Susac Syndrome: a case report

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  • corresponding author S. Puls - Augenklinik des Städtischen Klinikums, Karlsruhe
  • A. J. Augustin - Augenklinik des Städtischen Klinikums, Karlsruhe

Evidenzbasierte Medizin - Anspruch und Wirklichkeit. 102. Jahrestagung der Deutschen Ophthalmologischen Gesellschaft. Berlin, 23.-26.09.2004. Düsseldorf, Köln: German Medical Science; 2004. Doc04dogDO.17.08

The electronic version of this article is the complete one and can be found online at:

Published: September 22, 2004

© 2004 Puls et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.




SUSAC-Syndrome is characterized by branch retinal artery occlusion and microangiopathy of the inner ear and the brain in young subjects, mostly women. The pathogenesis is unknown.


A 22-years-old female patient developed a dramatic loss of hearing that mostly involved low frequencies. This was followed by cochleovestibular symptoms. Funducsopy and fluorescein angiography demonstrated bilateral branch artery occlusion. Brain MRI T2-weighted images revealed small multifocal hyperintensities of white and gray matter.


A first remission occurred some weeks after antithrombotic and immunosuppression by prednisolone, but some new branch occlusions were detected following withdrawal of the medication. Thus, systemic application of prednisolone has been continued for a couple of months (Ophthalmology 1998; 105:1038-44) followed by a stabilization of the clinical findings.


The symptoms are often misinterpretated as Multiple Sclerosis. Only a rare number of cases - less than 80 - have described in the literature. Treatment options are not standardized, but the disease seems to be self-limited after a fluctuating active phase. Its prognosis is good in most cases, the disease tends to be monophasic.